| Autor: |
Gallagher, Karen Patricia Domínguez, van Heerden, Willie, Said-Al-Naief, Nasser, Carlos, Roman, Arboleda, Lady Paola Aristizabal, Rodrigues-Fernandes, Carla Isabelly, Araújo, Anna Luíza Damaceno, Fonseca, Felipe Paiva, Pontes, Hélder Antônio Rebelo, Innocentini, Lara Maria Alencar Ramos, Romañach, Mário José, Vargas, Pablo Agustin, Lopes, Márcio Ajudarte, Santos-Silva, Alan Roger, Khurram, Syed Ali |
| Zdroj: |
Oral Surgery, Oral Medicine, Oral Pathology & Oral Radiology; Sep2022, Vol. 134 Issue 3, p354-366, 13p |
| Abstrakt: |
Objective: This systematic review aimed to identify the molecular alterations of head and neck rhabdomyosarcomas (HNRMS) and their prognostic values.Study Design: An electronic search was performed using PubMed, Embase, Scopus, and Web of Science with a designed search strategy. Inclusion criteria comprised cases of primary HNRMS with an established histopathological diagnosis and molecular analysis. Forty-nine studies were included and were appraised for methodological quality using the Joanna Briggs Institute Critical Appraisal tools. Five studies were selected for meta-analysis.Results: HNRMS predominantly affects pediatric patients (44.4%), and the parameningeal region (57.7%) is the most common location. The alveolar variant (43.2%) predominates over the embryonal and spindle cell/sclerosing types, followed by the epithelioid and pleomorphic variants. PAX-FOXO1 fusion was observed in 103 cases of alveolar RMS (79.8%). MYOD1 mutation was found in 39 cases of sclerosing/spindle cell RMS (53.4%). FUS/EWSR1-TFCP2 gene fusions were identified in 21 cases of RMS with epithelioid and spindle cell morphologies (95.5%). The 5-year overall survival rate of patients was 61.3%, and MYOD1 mutation correlated with significantly higher mortality.Conclusion: The genotypic profile of histologic variants of HNRMS is widely variable, and MYOD1 mutation could be a potential prognostic factor, but more studies are required to establish this. [ABSTRACT FROM AUTHOR] |
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